Chronic Infantile Neurological Cutaneous Articular syndrome (CINCA) (OMIM 607’115), also called Neonatal Onset Multisystem Inflammatory Disease (NOMID), is the most severe form of the Cryopyrin-Associated Periodic Syndromes (CAPS), a continuum of diseases with mutations of the CIAS1-gene also called NLRP3, coding for cryopyrin. Here, NLRP3 is linked to cryopyrin-associated periodic syndrome.